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Published on: March 26, 2019
Primary angiitis of the CNS
Rula A Hajj-Ali1, Aneesh B Singhal, Susanne Benseler
1Orthopaedic and Rheumatologic Institute, Cleveland Clinic, Cleveland, OH 44195, USA.
Significant advancements have been made in diagnosing primary angiitis of the CNS (PACNS) and its mimics over 30 years. While diagnostic accuracy has improved, challenges in understanding and managing PACNS persist in both children and adults.
Area of Science:
- Neurology
- Immunology
- Vascular Medicine
Background:
- Primary angiitis of the CNS (PACNS) is a rare condition affecting blood vessels in the brain and spinal cord.
- Understanding and diagnosis of PACNS have evolved significantly over the last three decades.
- Advances in neurological diagnostics have improved the recognition of PACNS and its differential diagnoses.
Purpose of the Study:
- To review the progress in understanding and clinical management of PACNS.
- To highlight advances in diagnostic approaches and the recognition of PACNS mimics.
- To identify remaining challenges in the diagnosis and management of PACNS.
Main Methods:
- Review of epidemiological, clinical, neuroradiagnostic, and laboratory findings.
- Analysis of case reports and clinical descriptions.
- Synthesis of advancements in diagnostic accuracy and mimic recognition.
Main Results:
- Increased recognition and aggressive diagnostic approaches for PACNS.
- Proliferation of case reports enhancing clinical and pathological descriptions.
- Improved diagnostic accuracy and identification of PACNS mimics through various findings.
Conclusions:
- Substantial progress has been achieved in the diagnosis and understanding of PACNS.
- Enhanced diagnostic tools and recognition of mimics have improved patient care.
- Despite advances, challenges in the comprehensive understanding and management of PACNS in all age groups remain.
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